Bleeding symptoms at disease presentation and prediction of ensuing bleeding in inherited FVII deficiency

Matteo Nicola Dario Di Minno; Alberto Dolce; Guglielmo Mariani; the STER Study Group; on behalf of

doi:10.1160/TH12-10-0740

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2013; 109(06): 1051-1059
DOI: 10.1160/TH12-10-0740

Blood Coagulation, Fibrinolysis and Cellular Haemostasis

Schattauer GmbH

Bleeding symptoms at disease presentation and prediction of ensuing bleeding in inherited FVII deficiency^{[
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Authors

Matteo Nicola Dario Di Minno

¹Department of Clinical and Experimental Medicine, Federico II University, Naples
Alberto Dolce

²Istituto Nazionale di Statistica, Palermo
Guglielmo Mariani

³Medical School, University of Ferrara, Ferrara, Italy

the STER Study Group

Abstract

Summary

Individuals with inherited factor VII (FVII) deficiency display bleeding phenotypes ranging from mild to severe, with 30% of patients having always been asymptomatic (non-bleeding). In 626 FVII-deficient individuals, by analysing data from the International Factor VII (IF7) Registry and the Seven Treatment Evaluation Registry (STER), we determined whether bleeding type at disease presentation and FVII coagulant activity (FVIIc) predict ensuing bleeds. At disease presentation/diagnosis, 272 (43.5%) individuals were non-bleeding, 277 (44.2%) had minor bleeds, and 77 (12.3%) had major bleeds. During a median nine-year index period (IP) observation, 87.9% of non-bleeding individuals at presentation remained asymptomatic, 75.1% of minor-bleeders had new minor bleeds, and 83.1% of major-bleeders experienced new major bleeds. After adjusting for FVIIc levels and other clinical and demographic variables, the relative risk (RR) for ensuing bleedings during the IP was 6.02 (p <0.001) and 5.87 (p <0.001) in individuals presenting with major and minor bleeds, respectively. Conversely, compared to non-bleeding individuals, a 10.95 (p = 0.001) and 28.21 (p <0.001) RR for major bleedings during the IP was found in those with minor and with major bleeds at presentation, respectively. In conclusion, in FVII deficiency, the first major bleeding symptom is an independent predictor of the risk of subsequent major bleeds.

Keywords

Asymptomatic individuals - minor bleeds - major bleeds - factor VII deficiency

Full Text

References

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Bleeding symptoms at disease presentation and prediction of ensuing bleeding in inherited FVII deficiency[ * ]

Authors

Bleeding symptoms at disease presentation and prediction of ensuing bleeding in inherited FVII deficiency^{[
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]}